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Low Anorectal Malformation

Presentation

Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication. [ncbi.nlm.nih.gov]

Female Demographics and Presentation Table 2. Male Demographics and Presentation Careful physical examination confirmed the diagnosis in all the cases. Typically the perineum was raw and irritated. [pediatrics.aappublications.org]

Although, female ARM and male children with low ARM may present late, almost all male patients with high ARM present within first few days of life. [atmph.org]

On perineal examination, an anocutaneous fistula with meconium was present between the scrotum and well-formed anal pit [Figure 1]. Erect abdominal X-ray revealed pneumoperitoneum [Figure 2]. [jcnonweb.com]

Entire Body System

  • Surgical Procedure

    This surgical procedure creates a stoma, an opening on the abdomen through which stool passes. [childrenscolorado.org]

    The colostomy is a surgical procedure that brings a segment of the large intestine out through an opening called a stoma that is made in the abdominal wall; this is a temporary situation and the colostomy is closed a few weeks after pull-through surgery [chp.edu]

    It may take several years to determine the final outcome of the surgical procedure. Specialized diets and medications may be needed to help your child have regular bowel movements and to reduce the chance of incontinence or soiling. [eapsa.org]

    All the surgical procedures were uneventful and at present the patient is doing well and is on regular follow-up. [jemds.com]

    Selecting the one- or three-step surgical procedure to treat the disease, especially in female neonates with rectovestibular fistula, is a subject of debate. This study aims to compare the advantages and disadvantages of these two methods. [afrjpaedsurg.org]

  • Asymptomatic

    The baby is asymptomatic on regular follow-up. [jcnonweb.com]

  • Developmental Disorder

    けいれん(痙攣)性疾患 cortex皮質 craniosynostosis頭蓋骨[縫合早期]癒合症 d [de la Tourette] combined vocal and multiple motor tic disorder音声および多発運動性の両者を含むチック障害、トゥレット障害 deafnessろう(聾)、聴覚消失[症] decerebrate rigidity除脳硬直 decorticate rigidity除皮質硬直 degeneration変性、変質 Denver developmental [tokyo-med.ac.jp]

Gastrointestinal

  • Failure to Thrive

    Failure to thrive Colonic atresia Enterocolitis Table 2. [jpss.eu]

Neurologic

  • Tic Disorder

    けいれん(痙攣)性疾患 cortex皮質 craniosynostosis頭蓋骨[縫合早期]癒合症 d [de la Tourette] combined vocal and multiple motor tic disorder音声および多発運動性の両者を含むチック障害、トゥレット障害 deafnessろう(聾)、聴覚消失[症] decerebrate rigidity除脳硬直 decorticate rigidity除皮質硬直 degeneration変性、変質 Denver developmental [tokyo-med.ac.jp]

  • Behavior Problem

    However, certain groups of parents are more at risk, i.e., parents with older, incontinent sons and parents with children exhibiting behavioral problems. [springerlink.com]

Urogenital

  • Hematuria

    Benign Familial Hematuria 500 Renal Tubular Acidosis 501 Nephrogenic Diabetes Insipidus 506 Renal Vascular Thrombosis 508 Urolithiasis 510 HemolyticUremic Syndrome 513 Syndrome of Inappropriate Secretion of Antidiuretic Hormone 514 Chronic Renal Failure [books.google.com]

Treatment

The results of the conservative treatment were comparable with that of the surgical treatment. Here, we presented the case of a 55-year-old man. Megarectosigmoids have been rarely documented in patients of this age. [e-fjs.org]

Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication. [ncbi.nlm.nih.gov]

Our renowned surgeons and faculty continue to push the frontiers of surgical science through innovative treatment and leading-edge research, changing lives and the future of surgical treatment, each and every day. [medicine.yale.edu]

The ultimate judgement regarding a particular clinical procedure or treatment must be made by the clinician in the light of the clinical data presented by the patient and the diagnostic or treatment options available. [uhs.nhs.uk]

Prognosis

Of the patients, 83% showed good prognosis and 15% had fair and poor prognosis. Our findings are similar. In the study of Hassink, there was a positive correlation between prognosis improvement and increasing age 15. [journals.lww.com]

Patients with this defect have all the necessary elements to be continent and have an excellent functional prognosis. [slideplayer.com]

These studies help to establish initial treatment and prognosis. How is ARM treated? All children with an ARM need at least one operation called an anorectoplasty or “pull-through”. [chp.edu]

Nonetheless, the prognosis of affected patients is far from excellent, as some degree of constipation is a frequent complaint in the long-term follow-up. Constipation in "low" ARM has been reported in 42%-70% of cases. [ncbi.nlm.nih.gov]

Despite this, the prognosis after surgery is very good, though children who’ve had this condition may suffer from constipation or urinary incontinence. [pullthrough.org]

Etiology

2016/5/4/274/194177 Introduction Neonatal colonic perforations are rare and even rarer are those which complicate anorectal malformations (ARMs). [1], [2] The commonly described etiologies of colonic perforations are intestinal atresias, Hirschsprung's [jcnonweb.com]

Vascular accidents occurring in utero are most commonly implicated in the etiology of CA; whereas, the etiology of ARM is widely considered multifactorial and may include participation of recessive genes (Nazer et al. 2000 ;Mo et al. 2001 ;Shinha et al [springerplus.springeropen.com]

Head Trauma 234 Sepsis and Septic Shock 242 Nonpolio Enteroviruses 332 Respiratory Syncytial Virus 334 Rhinoviruses 336 Roseola and Human Herpesviruses 337 Rubella 338 Rubeola 339 VaricellaZoster Virus 342 Viral Gastroenteritis 344 Disorders of Unknown Etiology [books.google.com]

BACKGROUND The etiology and pathogenesis of anorectal malformations (ARM) are multifactorial, poorly understood and controversial. [research.unipd.it]

Two lateral folds of cloacal tissue join the urorectal septum to complete the separation of the urinary and rectal tracts. 4 5 Imperforate anus Includes agenesis and atresia of the rectum and anus Etiology: unknown Incidence: 1 in 4,500 SEX: 60% male [slideplayer.com]

Epidemiology

"Genetics, Pathogenesis and Epidemiology of Anorectal Malformations and Caudal Regression Syndrome". In Holschneider, Alexander Matthias; Hutson, John M. (eds.). [en.wikipedia.org]

Springer, Berlin Heidelberg New York, pp 3–15 Google Scholar Harris J, Källén B, Robert E (1995) Descriptive Epidemiology of Alimentary Tract Atresia. [link.springer.com]

Epidemiology: Anorectal malformations (ARM) occurs in 1 baby in every 5,000 live births. It is equally seen in boys and girls. Figure 1: Picture of a baby's bottom without an anal opening. Picture courtesy of MJArca 11/2016. [eapsa.org]

Anal atresia, vertebral, genital and urinary tract anomalies: A primary polytopic developmental field defect identified through an epidemiological analysis of associations. Am J Med Genet 2000;13:95(2):169-73. 10. [pjms.com.pk]

[…] defects were seen less commonly in our patients. [1], [2], [17] Postoperative wound infection occurred in 3% of the patients, major source being contamination during colostomy as reported by others also. [7], [10], [18] This report gives an outline of the epidemiological [jiaps.com]

Pathophysiology

[…] surgery for low ARM can be done at the same time. [1] The overall mortality of perforated ARM is 19%. [1] Sepsis and disseminated intravascular coagulation were frequent causes of death. [1] Early diagnosis of ARM in neonates, better understanding of the pathophysiology [jcnonweb.com]

It is likely that other pathophysiological “triggers” tip the balance in favour of heavy soiling. Rapid colonic transit may be one such trigger. [intechopen.com]

Keywords: constipation, paediatric colorectal, Hirschsprung disease, anorectal malformation, VACTERL Hirschsprungs disease Definition and pathophysiology Hirschprungs disease (HD) is a congenital condition characterised by the absence of ganglion cells [jpss.eu]

Prevention

Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication. [ncbi.nlm.nih.gov]

This should be preventable by passing the dilator daily for the first few months of life. In the longer term constipation may be a problem but this can usually be managed by diet or medication. [uhs.nhs.uk]

In this case anal dilation will be all that’s necessary and surgery can be prevented, unless the anus is in the wrong place. [pullthrough.org]

Thus, the posterior muscle complex surrounds the rectum and helps prevent rectal prolapse. [22] (See the images below.) Operative view of posterior sagittal anoplasty in newborn with rectoperineal fistula. [emedicine.medscape.com]

We provide a Bowel Management Program, where we work closely with you and your child to assess function and provide a way for your child to stay clean of stool and prevent accidents. Learn More > [intermountainhealthcare.org]

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